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[克雅氏病早期深部白质中的多发孤立性病变。病例报告]

[Multiple solitary lesions in deep white matter in the early stage of Creutzfeldt-Jakob disease. A case report].

作者信息

Nomoto K, Murahashi M, Jimi T, Wakayama Y

机构信息

Department of Medicine, Showa University Fujigaoka Hospital, Yokohama.

出版信息

Rinsho Shinkeigaku. 1997 Jun;37(6):528-31.

PMID:9366185
Abstract

A 56-year-old housewife was admitted to our hospital because of involuntary movement on her left arm. Her neurological examination on admission showed mild weakness of her left arm and cerebellar ataxia. She developed periodic synchronous discharge on electroencephalogram and her cerebrospinal fluid revealed elevated level of neuron-specific enolase. Thereafter she developed dementia, followed by apallic state and diagnosed as having Creutzfeldt-Jakob disease (CJD). Interestingly, her MRI on admission revealed multiple solitary lesions in deep cerebral white matter, which were detected as high signal intensity by T2 weighed image. A few months later, these lesions tended to extend, and finally fused around the lateral ventricle in parallel with remarkable cortical atrophy. We excluded other diseases such as cerebrovascular disorders. Finally, we concluded the white matter change seen from early stage in this case may be the lesion associated with CJD, and the CJD case with early white matter changes has been seldomely described.

摘要

一名56岁的家庭主妇因左臂不自主运动入院。入院时神经系统检查显示左臂轻度无力及小脑共济失调。脑电图显示周期性同步放电,脑脊液中神经元特异性烯醇化酶水平升高。此后她出现痴呆,继而进入植物状态,被诊断为克雅氏病(CJD)。有趣的是,入院时她的MRI显示大脑深部白质有多个孤立性病灶,在T2加权像上呈高信号强度。几个月后,这些病灶有扩展趋势,最终在侧脑室周围融合,同时伴有明显的皮质萎缩。我们排除了脑血管疾病等其他疾病。最后,我们得出结论,该病例早期出现的白质改变可能是与CJD相关的病变,而早期有白质改变的CJD病例鲜有报道。

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